Pyoderma gangraenosum, a rare, but potentially fatal complication in paediatric oncology patients.

Pyoderma gangraenosum (PG) is a serious chronic, ulcerative skin disorder afflicting both adults and children. As PG is often associated with systemic diseases (>50%) such as inflammatory bowel disease, rheumatoid arthritis or haematological disorders, it requires a multidisciplinary approach. This disorder is not commonly reported in paediatrics; therefore children with PG represent a particularly difficult diagnostic challenge. Clinical diagnosis is often delayed and PG is only considered after eliminating other causes of cutaneous ulcers. We report a 4-year-old boy with secondary myelodysplastic syndrome following treatment for acute lymphoblastic leukaemia who presented with a massive inflammatory, ulcerative proliferation of the lower lip which was diagnosed as PG. We have reviewed the literature with reference to diagnostic criteria and treatment options of this disorder that is particularly rare in childhood.

Hepatic failure with neonatal tissue siderosis of hemochromatotic type in an infant presenting with meconium ileus. Case report and differential diagnosis of the perinatal iron storage disorders.

We report on a female preterm infant with hepatic failure and neonatal tissue siderosis of hemochromatotic type diagnosed by using both histochemistry and atomic absorption spectroscopy. The infant presented with meconium ileus, signs of rapidly progressive hepatic failure, and hyperferritinemia (7132 ng/ml). Despite surgery and intensive care the infant died 32 days after birth. Postmortem examination showed a wrinkled liver with extensive collapse of the hepatic architecture and regenerating nodules as well as hepatic and extrahepatic iron accumulation of hemochromatotic type, sparing the reticuloendothelial system. Atomic absorption spectroscopy confirmed an increase in the iron content of various organs: liver, heart, pancreas, oral salivary gland, kidney, and adrenal gland. The increase in the iron content of various organs was determined by comparing the analysis of the propositus with those of 5 gestationally age-related preterm infants who had died in the intensive care unit: 2 died of meconium aspiration syndrome, the other 3 of hyaline membrane disease, bronchopulmonary dysplasia, and immaturity, respectively. We also compared the analysis of 15 fetuses having a a condition predisposing to iron accumulation (trisomy 21, trisomy 18, cytomegalovirus, amnion infection syndrome, Rhesus- and ABO-incompatibility, congenital hemolysis, anti-phospholipid syndrome, congenital heart disease). Delta F508, the most frequent mutation seen in cystic fibrosis patients, was excluded by gene sequencing. Different noxae causing iron accumulation in the neonatal period have led to the statement that neonatal hemochromatosis may collect different etiologies, such as metabolic disorders, infections, chromosomal aberrations, and immunological disorders. In this study, we report the singular evidence of neonatal iron accumulation of hemochromatotic type in an infant presenting with meconium ileus and propose a classification of the neonatal disorders associated with iron accumulation.

Vascular tracheobronchial compression syndromes-- experience in surgical treatment and literature review.

Between January 1988 and December 1997 a total of 22 patients (age: 8 days-46 years) were operated for vascular airway compression syndromes with respiratory insufficiency. Vascular anomalies in tracheal compression were double aortic arch in 7 patients, (2 previously operated elsewhere), right aortic arch + left ligamentum arteriosum in 1, and pulmonary artery sling in 3. Three of these patients had secondary long-segment tracheomalacia. Compression of trachea and a main bronchus existed in 2 patients with right aortic arch + left ligamentum. Isolated main bronchus obstruction was present in 9 patients (abnormal insertion of ligamentum arteriosum in 1, status post (s.p.) previous operation for PDA in 4, s. p. surgery for coarctation in 1, right aortic arch + left ligamentum arteriosum in 2, and right lung aplasia + left ligamentum in 1). 3 of these cases had secondary long-segment bronchomalacia. All patients had a complex respiratory anamnesis [long-term intubation in 7, s.p. tracheostomy in 2 (over 3 months - 3 years), and progressive respiratory insufficiency in 13). In tracheal compression, surgical correction included transsection of the underlying ring or sling components (with additional anterior aortic arch translocation in 5 patients resection-reimplantation of left pulmonary artery in 3, segmental tracheal resection in 1, and external tracheal suspension in 2). In the 2 cases with compression of the trachea and a main bronchus, aortic "extension" by a prosthetic tube was necessary. In isolated main bronchus obstruction, surgical decompression basically consisted of transsection of the ligamentum arteriosum or resection of its scarry remnant forming the "corner point" of a compression between aorta and pulmonary artery. In 3 patients with secondary long-segment malacia, additional external bronchus suspension was performed. Effective decompression and re-expansion of the airway segment concerned was achieved, and was demonstrated by intraoperative endoscopy in all patients. There were 3 postoperative deaths (sepsis 2; massive, irreversible edema of the tracheal mucosa 1). Of the 19 surviving patients 16 could be extubated between the 1st and 17th (mean = 7.5) postoperative day. In 1 case the preoperative long-term tracheostomy had to be left in place for inoperable additional laryngeal stricture. 2 patients had to be reoperated (segmental cervical tracheal resection after 5 months for primary long-term intubation-related subglottic stenosis in 1, esophageal decompression for residual dysphagia after 57 months related to a traction phenomenon at the right descending aorta in the other), both with gratifying results. In all other patients clinical, endoscopic, and radiographic examinations (follow-up = 2 months - 6 years) demonstrate good results.

Refractory congenital ascites as a manifestation of neonatal sialidosis: clinical, biochemical and morphological studies in a newborn Syrian male infant.

A Syrian newborn with coarse facies, hepato-splenomegaly, and refractory ascites is reported. Examination of the ascitic fluid showed vacuolated lymphocytes and thin-layer chromatography of urinary oligosaccharides revealed an abnormal pattern indicative of sialidosis. Despite intensive care, the baby died of respiratory insufficiency 28 days after birth. In cultured skin fibroblasts an increase of the incorporation of [14C]methylamine pointed to excessive lysosomal storage and the demonstration of an isolated deficiency of alpha-N-acetylneuraminidase (sialidase) led to the diagnosis of a sialidosis. At postmortem examination, foam cells were found mostly in bone marrow, liver, and brain. To date very few cases of neonatal sialidosis have been reported, and, to the best of our knowledge, this is the first child with neonatal sialidosis from Syria and the first case of neonatal sialidosis studied by the [14C]methylamine incorporation assay.

Impaired deformability of erythrocytes and neutrophils in children with newly diagnosed insulin-dependent diabetes mellitus.

AIMS/HYPOTHESIS: Abnormal rheological properties of erythrocytes, leucocytes and plasma may have a role in the development of diabetic microangiopathy. We hypothesized that changed haemorrheological variables may already be found in children with onset diabetes. METHODS: Erythrocyte deformation (rheoscope), neutrophil deformation (micropipette), erythrocyte aggregation, blood and plasma viscosity were measured in 15 children with insulin-dependent diabetes mellitus before initiation of insulin treatment and 4 to 6 weeks later, 15 diabetic children treated with insulin for 5 to 8 years, 15 healthy children and 15 healthy adults. RESULTS: At a low shear stress of 0.6 Pa, erythrocyte deformation was decreased in the diabetic children before (-28%), after 4 to 6 weeks (-22%) and after 5 to 8 years (-17%) of insulin treatment compared with healthy children. More active neutrophils were counted in the untreated diabetic children (9 +/- 6%) than in healthy children (3 +/- 2%). Deformability of passive neutrophils was greatly decreased in the children with onset diabetes and moderately reduced in the diabetic children who were treated with insulin. Neutrophil deformation (r = -0.52) and erythrocyte deformation at 0.6 Pa (r = -0.62) were inversely related to haemoglobin A1c. Haematocrit and blood viscosity were increased in the untreated children and in the children treated with insulin for 5 to 8 years. Plasma viscosity and erythrocyte aggregation were similar in the three groups of children. CONCLUSION/INTERPRETATION: Decreased erythrocyte deformation at low shear force, increased count of active neutrophils and impaired deformability of passive neutrophils may increase the risk for acute cerebro-vascular complications in children with uncontrolled insulin-dependent diabetes mellitus.

Clinical symptoms, biochemical studies and therapeutic approaches in a sibship with a new congenital tubulopathy.

UNLABELLED: We report on two siblings suffering from a new congenital tubulopathy. Following normal pregnancies not complicated by polyhydramnios, severe renal losses of potassium, chloride, sodium and magnesium occurred in the first weeks after birth. Calcium metabolism was not affected. The distal tubular chloride reabsorption was considerably decreased in the two siblings (0.25 and 0.28, respectively). Secondary hyperaldosteronism, activation of the kallikrein-kinin system and elevated urinary prostaglandin excretion were observed. The effects of indomethacin, spironolactone and captopril on symptoms, electrolyte wasting, activation of renin-angiotensin-aldosterone and kallikrein-kinin system and prostaglandin synthesis were studied. In spite of persisting elevation of prostaglandin synthesis, captopril decreased electrolyte wasting, polyuria and hyperaldosteronism most effectively. CONCLUSION: We delineate an apparently new disorder characterized by a postnatal onset, an extremely decreased chloride reabsorption with extensive hyperchloriduria and hypermagnesiuria in the presence of normal calcium metabolism. The disorder can be distinguished from other tubulopathies with hypokalaemic alkalosis.

Wolf-Hirschhorn syndrome: case report and review of the chromosomal aberrations associated with diaphragmatic defects.

A female fetus showing severe growth retardation was delivered at 31 weeks of gestation because of fetal distress. At birth, the infant showed bradycardia and no spontaneous breathing. Although high frequency oscillatory ventilation was started, severe asphyxia persisted and the infant died of respiratory insufficiency. At the autopsy, the propositus showed microcephaly, prominent glabella, broad bridge of the nose, ocular hypertelorism, poorly differentiated and low-set ears, bilateral palatoschisis, and micrognathia. Midline closure defects of the cervical spine bodies, lower jaw, and skull base were seen at postmortem radiography. An extreme hypoplasia of both lungs, a large defect of the left diaphragm with upward displacement of viscera, and multiple cortical cysts in both kidneys were seen at postmortem examination. Karyotyping revealed a chromosomal imbalance with 46, XX, del(4) (pter-->13), characterizing the Wolf-Hirschhorn syndrome. Because diaphragmatic defects can occur in association with specific recognizable patterns of human malformation careful pathologic and genetic workup of all affected infants in crucial for accurate genetic counseling.

Meconium aspiration syndrome complicated by massive intravascular thrombosis.

Fetal aspiration of meconium in amniotic fluid during fetal distress by newborn infants can induce the meconium aspiration syndrome (MAS), a form of neonatal respiratory distress. Should this event occur, admission to a Neonatal Intensive Care Unit and vigorous airway management and monitoring are required. We present a term gestation resulting in MAS complicated by a massive intravascular thrombosis. Despite airway management considered appropriate, the infant developed respiratory distress a few hours after birth and died 5 days later. Postmortem examination showed a diffuse alveolar damage of the lungs with alveoli filled with meconium and amniotic epithelial cells as well as disseminated thrombi in the pulmonary vascular tree, portal system, suprahepatic veins, and peripheral arterial vascular tree.

External stabilization of long-segment tracheobronchomalacia guided by intraoperative bronchoscopy.

BACKGROUND: Symptomatic obstruction of long-segment tracheal or bronchial portions either related to congenital instability or secondary to vascular compression are rare malformations, which remain difficult to manage. A method of external tracheal or bronchial stabilization is described. METHODS: From July 1992 to April 1995, 7 children (age range, 4 months to 4 years; mean age, 19 months) and 1 adult (age, 46 years) were operated on for severe respiratory insufficiency. In 4 cases of congenital tracheal instability, 2 children had associated type IIIb esophageal atresia. Both children with esophageal atresia had previous operations (two and three times, respectively): 1 child had aortopexy and division of a patent ductus arteriosus and another child had distal tracheal resection elsewhere, both without relief of malacia. All children were intubated and ventilated since birth for 11 to 15 months. Secondary tracheobronchomalacia due to vascular compression was seen in 4 patients caused by double aortic arch (n = 2) and persisting ligamentum arteriosum after previous ligation of a patent ductus arteriosus (n = 2), with 1 child ventilated thereafter for 5 months. Operation was performed with the aid of extracorporeal circulation in all patients but 1, and consisted of transection of vascular rings and persistent ligamentum Botalli (n = 5), closure of multiple ventricular septal defects (n = 1) and extensive mobilization of the tracheobronchial tree as well as the great arteries. External stabilization of the severely dysplastic distal trachea (n = 6) or left main bronchus (n = 2) was achieved by suspending the malacic segment within an oversized and longitudinally opened ring-reinforced polytetrafluoroethylene prosthesis. Multiple plegeted sutures were placed extramucosally to the dysplastic tracheal wall and the dyskinetic pars membranacea, as well as to the polytetrafluoroethylene prosthesis in a radial orientation. Guided by simultaneous video-assisted bronchoscopy, reexpansion of the collapsed segments was achieved by gentle traction on the sutures while tying. RESULTS: Stenosis-free tracheobronchial reexpansion was achieved in all patients, as seen on repeated bronchoscopies during hospitalization and thereafter. All patients were extubated within 1 to 12 days after the operation. There was one late death, unrelated to the procedure, in a 31-month-old child 20 months after the operation. All other patients are free of stridor and in excellent clinical condition 21 to 54 months (mean, 38 months) thereafter. CONCLUSIONS: The presented method of bronchoscopically guided external tracheobronchial suspension within a ring-reinforced polytetrafluoroethylene prosthesis immediately relieves severe malacia of the trachea or main bronchi in infants as well as adults without necessitating resection. Midterm preliminary data suggest that growth potential of the affected segment exists within the oversized polytetrafluoroethylene prosthesis.

Effects of high-frequency oscillatory ventilation on circulation in neonates with pulmonary interstitial emphysema or RDS.

OBJECTIVE: Mechanical ventilation may impair cardiovascular function if the transpulmonary pressure rises. Studies on the effects of high-frequency oscillatory ventilation (HFOV) on cardiovascular functions have yielded conflicting results. This study was done to compare alterations in left ventricular output and blood flow velocities in the anterior cerebral artery, internal carotid artery, and celiac artery using a Doppler ultrasound device before and 2 h after initiating HFOV in neonates with respiratory distress syndrome (RDS) or pulmonary interstitial emphysema (PIE). DESIGN: Prospective clinical study. SETTING: Neonatal intensive care unit in a perinatal center. PATIENTS: 18 critically ill infants (postnatal age 47 +/- 12 h; mean +/- SD) were studied before and during HFOV (piston oscillator). Indications for HFOV were severe respiratory failure due to PIE (n = 10) and severe surfactant deficiency (RDS, n = 8). In the RDS group, gestational age was 27 +/- 6 weeks (range 26-31 weeks) and birth-weight 1620 +/- 380 g (range 850-1970 g). In the PIE group, gestational age was 28 +/- 2 weeks (range 26-36 weeks) and birth-weight 1740 +/- 470 g (range 890-2760 g). MEASUREMENTS AND MAIN RESULTS: During HFOV, mean airway pressure was maintained at the same level as during intermittent mandatory ventilation in both groups (RDS, 12 +/- 2 cmH2O; PIE, 10 +/- 2 cmH2O). Compared to intermittent mandatory ventilation, several of the 12 parameters studied changed significantly (p < 0.004) during HFOV. In the RDS group, the partial pressure of oxygen in arterial blood/fractional inspired oxygen (PaO2/FIO2) ratio increased from 56 +/- 9 to 86 +/- 7 and partial pressure of carbon dioxide in arterial blood (PaCO2) decreased from 49 +/- 4 to 35 +/- 3 mmHg. In the PIE group, PaO2/FIO2 ratio increased from 63 +/- 8 to 72 +/- 7 and PaCO2 decreased from 63 +/- 7 to 40 +/- 5 mmHg. In the PIE group, heart rate decreased (135 +/- 15 before HFOV vs 115 +/- 14 min-1 during HFOV) and mean systolic blood pressure increased (before 43 +/- 4 vs 51 +/- 4 mmHg during HFOV) significantly, whereas these parameters did not change in the RDS group. Left ventricular output increased significantly in the PIE group (210 +/- 34 before vs 245 +/- 36 ml/kg per min during HFOV; p < 0.004), but not in the RDS group (225 +/- 46 before vs 248 +/- 47 ml/kg per min during HFOV; k < 0.05). Shortening fraction and systemic resistance did not change in either group. In the PIE group, mean blood flow velocities in the internal carotid artery (+59%), anterior cerebral artery (+65%) and celiac artery (+45%) increased significantly but did not change in the RDS group. CONCLUSIONS: The results show that HFOV as used in this study, improves oxygenation, CO2 elimination, and circulation in infants with RDS and PIE. However, systemic, cerebral, and intestinal circulation improved more in neonates with PIE than in those with RDS. This may be due to higher pulmonary compliance in infants with PIE when compared to those with RDS.

Alternative pathway activation of the complement system in preterm infants with early onset infection.

The increased incidence of infection in preterm neonates has been related in part to their relative deficiency of most complement components, because complement is known to participate in the defense against bacterial and viral infections. In a prospective study, complement activation products were determined in 52 preterm infants. Twenty preterm infants suffered from proven early onset infection, 11 infants were presumed to suffer from infection, which could not be confirmed. Twenty-one preterm infants without infection or perinatal asphyxia formed the control group. EDTA plasma was obtained within the first 6 h after birth, and follow-up examinations were done in 15 patients with proven infection during the next 24 h. The complement activation products C3a-desArg, C3bBbP, and sC5b-9 were measured with enzyme immunoassay systems. In preterm neonates with early onset infection, a significant elevation of C3a-desArg was found in the very early course of the disease. C3a-desArg generation resulted from alternative pathway activation as shown by a concurrent increase of C3bBbP concentration. In addition, significantly higher concentrations of sC5b-9 predicted infection in the first few hours after birth. Thus, despite very low levels of native complement proteins, preterm babies are able to generate remarkable amounts of activation products of the complement cascade. The elevation of these activation products preceded by hours significant changes of routine laboratory markers of infection, such as leukocyte count, differential blood count, and C-reactive protein. Thus they might help to identify preterm neonates with severe systemic infection earlier than other laboratory parameters.

[High frequency jet ventilation during tracheal resection in children and infants]. / Hochfrequenz-Jet-Beatmung während Trachearesektion bei Kindern und Säuglingen.

Between 1986 and 1996, 16 infants and children less than 11 years of age (m = 11, f = 5) underwent resections for acquired or congenital tracheobronchial stenoses. During this period, various techniques of total intravenous anaesthesia (TIVA) were employed (midazolam, fentanyl, pancuronium; propofol, fentanyl, pancuronium). During the phase of dividing the airways, high-frequency-jet ventilation (HFJV) into the trachea or the main bronchi by 8-12Fr catheter(s) was applied for 10-75 min with driving pressures between 0.3-1.8 bar, frequencies between 100-200/min, I:E ratio between 1:4-1:1, and FjetO2 1.0. Catheter position was controlled visually, gas exchange was monitored by pulse oximetry and blood gas analysis. There were two incidents of transient hypoxaemia (paO2 less than 60 mmHg), and 4 cases of hypercapnia (paCO2 more than 45 mmHg). No complications due to the HFJV-catheter technique, such as barotrauma or aspiration were seen. All children were kept postoperatively on a ventilator due to swelling of the airway anastomosis. In 5 children ventilator treatment exceeded 7 days, 3 children were discharged tracheostomised. These observations serve to confirm that HFJV is capable of maintaining gas exchange during tracheal resection in infants and children, if the following prerequisites are met: 1. Tracheobronchial pathology suitable for poststenotic placement of jet catheter. 2. No respiratory impairment by parenchymal pathology. 3. Monitoring by continuous visual control of respiratory mechanics, pulse oximetry and blood gas analysis. Cardiopulmonary bypass should be applied if airway pathology precludes safe placement of jet catheters, or in the presence of parenchymal respiratory failure.

[Bronchopulmonary dysplasia. Retrospective analysis of various forms of treatment and development of a staged therapeutic plan]. / Bronchopulmonale Dysplasie. Retrospektive Analyse verschiedener Behandlungsformen und Entwurf eines therapeutischen Stufenplans.

50 premature infants with bronchopulmonary dysplasia (BPD) were treated in the Perinatal Center of the University of Heidelberg from January 1990 to December 1992. Gestational age was 24-31 weeks and birthweight was 500 to 1430 grams. 27 infants received dexamethasone only and 14 were initially given dexamethasone followed by beclomethasone inhalation. Nine infants without assisted ventilation were only treated with inhaled beclomethasone. Infants with fluid intake > 150 ml/kg/d and < or = 150 ml/kg/d were analysed separately. Extubation in ventilated infants was possible 1 to 29 days after the beginning of dexamethasone treatment. Most infants who were not ventilated any more could be weaned from oxygen during the period of dexamethasone treatment. Inhaled beclomethasone allowed reduction in supplemental oxygen in all infants. Effects of treatment with dexamethasone and beclomethasone were similar in infants with fluid intake of < 150 ml/kg/d and > 150 ml/kg/d. Our data show that dexamethasone and inhaled beclomethasone improved the clinical course of BPD in premature infants. Fluid intake had no influence on clinical outcome. Based on our results, we suggest guidelines for the treatment of BPD.

[Rheologic changes in stored erythrocyte concentrates]. / Rheologische Veränderungen gelagerter Erythrozytenkonzentrate.

We studied the effect of two different preservation solutions on mean corpuscular volume (MCV), red cell deformability and flow in narrow tubes in red blood cell concentrates. Blood from 10 healthy blood donors was processed in parallel in SAG-M (S-RBC) as well as in PAGGS-M (P-RBC) in identical aliquots. Samples were studied at days 0, 7, 14, 28 and 42 of storage. MCV was determined using a Du Pont cell counter. Whole cell deformability was determined in a Myrenne Rheodyn. Viscosity reduction in narrow tubes was determined by means of capillary viscosimetry. P-RBC showed a constant MCV over the entire storage period. In contrast, MCV of S-RBC increased and MCHC decreased during storage. P-RBC showed similar deformability and viscosity reduction during storage, whereas deformability decreased and viscosity reduction became less pronounced for S-RBC. Our study shows superior rheological properties of P-RBC. Thus, PAGGS-M may provide better hemoglobin flux and oxygen transport to tissues than SAG-M.

Effect of Leboyer childbirth on cardiac output, cerebral and gastrointestinal blood flow velocities in full-term neonates.

The Leboyer birth method requires that the newly born infant is placed on the mother's abdomen and the cord is clamped when it stops pulsating. This investigation was done to study the effect of Leboyer childbirth on neonatal circulation during the first 5 days after birth. Hematocrit, blood viscosity, left and right ventricular output, and cerebral blood flow velocities in the arteria carotis interna, arteria cerebri anterior, and truncus coeliacus were studied in 15 full-term neonates with early (less than 10 seconds) cord clamping and 15 full-term neonates delivered according to Leboyer (cord clamping after 3 minutes) on day 1 (2 to 4 hours after birth) and day 5. The fetal placental blood volume decreased from 42 +/- 8 mL/kg (mean +/- SD) of neonatal body weight after early cord clamping to 19 +/- 7 mL/kg after Leboyer delivery. Neonatal blood volume, calculated from the fetal placental blood volume, was 32% higher in the Leboyer group compared with the early cord-clamped infants. In the infants with early cord clamping, hematocrit, and blood viscosity did not change significantly during the first 5 days. After Leboyer birth, the hematocrit rose from 0.51 +/- 0.05 in cord blood to 0.62 +/- 0.06 at 2 to 4 hours of age, thereby increasing blood viscosity by 32%. Stroke volume, heart rate, cardiac output, left-to-right shunt across the ductus arteriosus, and blood flow velocity in the truncus coeliacus were similar in both groups and did not change during the first 5 days.(ABSTRACT TRUNCATED AT 250 WORDS)

Effects of red cell transfusion on cardiac output and blood flow velocities in cerebral and gastrointestinal arteries in premature infants.

Anaemia may increase the risk of tissue hypoxia in preterm infants. The effect of transfusion on circulation was studied in 33 preterm infants with a mean (SD) gestational age of 29 (5) weeks (range 26-34), birth weight 1153 (390) g (range 520-1840), and postnatal age of 48 (21) days (range 19-100). Packed cell volume, blood viscosity (capillary viscometer), cardiac output, and cerebral blood flow velocities in the internal carotid artery, anterior cerebral artery, and coeliac trunk (Doppler ultrasound) were determined before and after transfusion of 10 ml/kg of packed red blood cells. Transfusion increased packed cell volume from a mean (SD) 0.27 (0.45) to 0.37 (0.48). Mean arterial blood pressure did not change while heart rate decreased significantly from 161 (14) l/min to 149 (12). Cardiac output decreased from 367 (93) ml/kg/min to 311 (74) due to decrease in stroke volume from 2.28 (0.57) ml/kg to 2.14 (0.46) and in heart rate. There was a significant increase in systemic red cell transport (cardiac output times packed cell volume) by 17%, systemic flow resistance (blood pressure to cardiac output ratio) by 23%, and blood viscosity by 33%. Vascular hindrance (flow resistance to blood viscosity ratio) did not change significantly, thereby suggesting that neither vasoconstriction nor vasodilation occurred with transfusion. After transfusion blood flow velocities decreased significantly in the anterior cerebral artery by 23%, in the internal carotid artery by 8%, and in the coeliac trunk by 12%. Red cell transport estimated as products of blood flow velocities times packed cell volume increased significantly by 25% in the internal carotid artery and by 21% in the coeliac trunk. These results indicate that red cell transfusion improved systemic oxygen transport as well as oxygen transport in the internal carotid artery and coeliac trunk.

Plasmapheresis in severe septic shock with disseminated intravascular coagulation.

An 18-year-old female with CNS relapse of acute lymphoblastic leukemia after previous complete remission of the disease underwent chemotherapy. Due to the therapy she suffered from profound suppression of bone marrow with consecutive thrombocytopenia and leukopenia. Despite prophylactic treatment, severe septicemia occurred with septic shock, hemolysis and disseminated intravascular coagulation (DIC). As the clinical course became uncontrollable by means of conventional therapy, including broad-spectrum antibiotics, substitution of fresh frozen plasma, antithrombin III and heparin therapy, plasma exchange was used as a rescue therapy. This method succeeded in effective replacement of clotting factors and normalization of coagulation, in removal of fibrinogen degradation products and probably of toxins and shock mediators. The patient recovered from shock.

Complement activation in newborn infants with early onset infection.

The complement system is an important element in host defense. Quantitative deficiencies of total hemolytic complement activity and decreased C3 levels were reported in sera from normal neonates. However, little is known about complement activation products in the newborn. In a prospective study, complement activation products were determined in 32 healthy term neonates, in 41 neonates with colonization of their mothers, in 15 colonized neonates, and in 10 neonates with early onset infection. In all newborns, EDTA plasma was obtained within the first 6 h of life. The anaphylatoxin C3a-desArg was determined with a novel ELISA using an MAb reacting with a neoepitope of C3a-desArg. C3bBbP (alternative pathway convertase) and C1rsC1-inactivator (activation product of classical pathway) were measured with double-sandwich ELISA. C3 was determined by radial immunodiffusion. Plasma concentrations of C3a-desArg were similar in healthy term neonates and healthy adults, whereas diminished C3 levels were observed in the newborn infants. There were no significant differences between healthy neonates, neonates with colonized mothers, and colonized neonates. In neonates with infection, a significant elevation of C3a-desArg was found at the onset of the disease, resulting from alternative pathway activation. In contrast, the C1rsC1-inactivator complex showed no significant differences among healthy, colonized, and infected neonates. The anaphylatoxin C3a mediates inflammatory reactions such as vasodilatation and an increase in microvascular permeability and might therefore play an important role in severe neonatal infection.

The effect of Leboyer delivery on blood viscosity and other hemorheologic parameters in term neonates.

OBJECTIVE: This study was done to compare postnatal alterations in blood viscosity, hematocrit value, plasma viscosity, red blood cell aggregation, and red blood cell deformability in term neonates undergoing both early umbilical cord clamping and delivery according to the Leboyer method. STUDY DESIGN: The umbilical cords of 15 healthy, term infants were clamped within 10 seconds of birth (early cord clamping), and 15 infants delivered according to the Leboyer method were placed on the mother's abdomen, and the umbilical cords were clamped 3 minutes after birth. Hemorheologic parameters were studied in umbilical cord blood at 2 hours, 24 hours, and 5 days from the time of delivery. RESULTS: The residual fetal placental blood volume decreased from 45 +/- 8 ml/kg (x +/- SD) after early cord clamping to 25 +/- 5 ml/kg after delivery by the Leboyer method. After Leboyer-method delivery, the hematocrit value rose from 48% +/- 5% at birth to 58% +/- 6% 2 hours after delivery, 56% +/- 7% at 24 hours, and 54% +/- 8% after 5 days. Blood viscosity in the Leboyer-method group increased by 32% within the first 2 hours but did not change significantly during the following 5 days. Plasma viscosity, red blood cell aggregation, and red blood cell deformability were not affected by the mode of cord clamping. CONCLUSIONS: Delivery by the Leboyer method leads to a significant increase in blood viscosity as a result of increasing hematocrit value, whereas other hemorheologic parameters are similar to those of infants with early cord clamping.